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Background and Aim: Pemphigoid gestationis (PG) is a rare skin disease of pregnancy. Given its incidence in pregnant women, physicians and especially obstetricians may not encounter this diagnosis in their entire career. We find this to be a major problem and there is an obligation to report it in as much detail as possible along with recommended treatments with proven efficacy., Case Report: We describe the case of a 27 year old patient who was referred to the dermatology department with severe dissemination of blisters in the 9th week of pregnancy. She was diagnosed with pemphigoid gestationis in her first pregnancy. High doses of corticosteroids were initiated but due to inadequate effect cyclosporine was added. The pregnancy was complicated with gestational diabetes. The patient gave birth in her 33rd week by caesarian section due to premature rupture of the membrane. Vesicles were seen on the newborn immediately after birth which diminished spontaneously over 2 weeks. Blisters were still seen on the patient 1 month after labor even with the combination of systemic corticosteroids with cyclosporine., Conclusion: PG is a rare dermatosis of pregnancy. The course of the disease can be severe, necessitating systemic therapy. As described in this patient, systemic corticosteroids may not be sufficient and adding another immunosuppressive treatment may be needed. If pemphigoid gestationis has occurred during a previous pregnancy it is advised to reconsider another pregnancy., Competing Interests: The authors report no conflicts of interest in this work.

Background: Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) may be similar morphologically but confer different maternal and fetal risks. Direct immunofluorescence is the gold standard test used to differentiate between the 2 diagnoses but is not always available., Objective: To develop and validate a clinical scoring system to differentiate PG from PEP., Methods: After developing a scoring system based on differentiating clinical factors reported in existing literature, we tested its diagnostic accuracy in a retrospective international multicenter validation study in collaboration with the European Academy of Dermatology and Venereology's Skin Diseases in Pregnancy Taskforce., Results: Nineteen pregnancies (16 patients) affected by PG and 39 pregnancies (39 patients) affected by PEP met inclusion criteria. PG had a mean score of 4.6 (SD, 2.5) and PEP had a mean score of -0.3 (SD, 2.0). The area under the curve was 0.93 (95% CI, 0.86-1.00). Univariate analysis revealed that almost all criteria used in the scoring system were significantly different between the groups (P < .05), except for skip pregnancy and multiple gestations, which were then removed from the final scoring system., Limitations: Small retrospective study., Conclusion: The Pregnancy Dermatoses Clinical Scoring System may be useful to differentiate PG from PEP in resource-limited settings., Competing Interests: Conflicts of interest Dr Murase has served as an advisor for LeoPharma, Sanofi-Genzyme, Eli Lilly, Regeneron, and UCB and has given disease-state nonbranded talks for Regeneron and UCB. Dr Maul is an employee of USZ and has served as an advisor and/or received speaking fees and/or participated in clinical trials sponsored by AbbVie, Almirall, Amgen, BMS, Celgene, Eli Lilly, LEO Pharma, Janssen-Cilag, MSD, Novartis, Pfizer, Pierre Fabre, Roche, Sanofi, UCB. Dr Lehman has served on the advisory board of Argenx. Other authors have no conflicts of interest to declare., (Copyright © 2023 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Adverse pregnancy outcomes (APO) occur in 35% of patients with pemphigoid gestationis (PG). No biological predictor of APO has been established yet., Objectives: To assess a potential relationship between the occurrence of APO and the serum value of anti-BP180 antibodies at the time of PG diagnosis., Methods: Multicentre retrospective study conducted from January 2009 to December 2019 in 35 secondary and tertiary care centres., Inclusion Criteria: (i) diagnosis of PG according to clinical, histological and immunological criteria, (ii) ELISA measurement of anti-BP180 IgG antibodies determined at the time of PG diagnosis with the same commercial kit and (iii) obstetrical data available., Results: Of the 95 patients with PG included, 42 had one or more APO, which mainly corresponded to preterm birth (n = 26), intrauterine growth restriction (IUGR) (n = 18) and small weight for gestational age at birth (n = 16). From a ROC curve, we identified a threshold of 150 IU ELISA value as the most discriminating to differentiate between patients with or without IUGR, with 78% sensitivity, 55% specificity, 30% positive and 91% negative predictive value. The threshold >150 IU was confirmed using a cross-validation based on bootstrap resampling, which showed that the median threshold was 159 IU. Upon adjusting for oral corticosteroid intake and main clinical predictors of APO, an ELISA value of >150 IU was associated with the occurrence of IUGR (OR = 5.11; 95% CI: 1.48-22.30; p = 0.016) but not with any other APO. The combination of blisters and ELISA values higher than 150 IU led to a 2.4-fold higher risk of all-cause APO (OR: 10.90; 95% CI: 2.33-82.3) relative to patients with blisters but lower values of anti-BP180 antibodies (OR of 4.54; 95% CI 0.92-34.2)., Conclusion: These findings suggest that anti-BP180 antibody ELISA value in combination with clinical markers is helpful in managing the risk of APO, in particular IUGR, in patients with PG., (© 2023 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.)

Background: Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) are pregnancy-related dermatoses. Definitive diagnosis often relies upon histopathology and direct immunofluorescence (DIF). PG is associated with fetal and neonatal risks, while PEP confers minimal risk., Objective: We aimed to compare histopathologic features to determine key differentiators., Methods: A retrospective cohort study of PG and PEP cases, with accompanying DIF, conducted from 1995 to 2020. Skin biopsies were examined independently in a blinded fashion by two dermatopathologists for a list of histopathological features., Results: Twenty-one cases of PG and 10 cases of PEP were identified. PG had significantly denser eosinophils than PEP (mean 155 vs. 48 cells/5 hpf; p < 0.018). PG was also noted to have eosinophilic spongiosis and eosinophils at the dermal-epidermal junction more frequently compared to PEP (80% PG vs. 10% PEP; p < 0.001). A mean cutoff value of 86 eosinophils and a mean optimal sensitivity and specificity of 81% and 83%, respectively, for eosinophils density's diagnostic power of PEP versus PG were achieved. Subepithelial separation was exclusively seen in PG (40% vs. 0%; p < 0.007)., Conclusion: Eosinophilic spongiosis, eosinophilic epitheliotropism, and dense superficial dermal eosinophils were diagnostic of PG. Given overlapping clinicopathologic features, however, DIF results with clinicopathologic correlation, remain the gold standard., (© 2022 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Pregnancy dermatoses are inflammatory skin disorders that occur during pregnancy or immediately postpartum. This heterogenous group of disorders includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, atopic eruption of pregnancy, and pustular psoriasis of pregnancy. In this article, we provide a comprehensive literature review of each condition focusing on nomenclature, epidemiology, pathogenesis, clinical presentation, diagnosis, differential diagnosis, maternal risk, fetal risk, and treatment. We aim to increase awareness and help clinicians recognize, diagnose, and manage these unique conditions., Competing Interests: Financial Disclosure: Miriam Keltz Pomeranz is a member of the scientific advisory board of Proctor&Gamble and is an author in UpToDate. Jaclyn Rosenthal Himeles did not report any potential conflicts of interest., (Copyright © 2022 by the American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Pemphigoid gestationis (PG), also known as gestational pemphigoid, as it is specifically associated with a pregnancy event, is among the rare pregnancy-related dermatoses, characterised by the formation of autoantibodies against Bullous Pemphigoid antigens 180 and 230 (BP180 and BP230), causing significant damage to the basement membrane of the skin, resulting in marked pruritus and blisters on the abdomen and extremities. Diagnosis of PG is basically made by the characteristic clinical picture and confirmed by immunofluorescence studies and histopathology of a skin biopsy. Treatment, just as for other autoimmune dermatoses, is achieved by corticosteroids with the risk of relapses in subsequent pregnancies. Fetal growth restriction and pre-maturity are potential fetal complications associated with the disease, hence the recommended combined antenatal care by a dermatologist as well as an obstetrician, however, this disease is unlikely to be a source of significant maternal morbidity or mortality., Competing Interests: Declaration of Competing Interest We declare no competing interests of the authors, (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Pregnancy has a substantial impact on the hormonal status of the organism, consequently influencing the physiology of the skin. This results in dermatoses that only occur during pregnancy, which can also improve or exacerbate pre-existing dermatoses. In this article, we explain the management of pregnancy-specific dermatoses : atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis, impetigo herpetiformis, and intrahepatic cholestasis of pregnancy. It is essential to clearly distinguish these different dermatoses as some of them, such as pemphigoid gestationis, impetigo herpetiformis and intrahepatic cholestasis of pregnancy, can have fetal consequences and as result, need to be closely monitored by the obstetricians., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Pruritus in pregnancy is a common and burdensome symptom that may be a first sign of a pregnancy-specific pruritic disease (atopic eruption of pregnancy, polymorphic eruption of pregnancy, pemphigoid gestationis, and intrahepatic cholestasis in pregnancy) or a dermatosis coinciding with pregnancy by chance. Despite its high prevalence, pruritus is often underrated by physicians, and data regarding the safety profiles of drugs for pruritus are very limited. In this review, we illustrate the epidemiology, possible pathophysiology, clinical characteristics, and diagnostic workup of various pregnancy-related diseases and discuss antipruritic treatments. The prevalence of pruritus in pregnancy demonstrates the importance of symptom recognition and the need for an holistic approach, taking into account both the potential benefits for the patient and the potential risks to the fetus., (© 2022. The Author(s).)

Background: Women with rashes or itchy skin during pregnancy will often present initially to the general practitioner. Knowledge of the specific dermatoses of pregnancy will assist in diagnosis, management and, importantly, facilitation of timely escalation of care of conditions that can potentially affect the fetus., Objective: The aim of this article is to provide a diagnostic framework for approaching a pruritic rash during pregnancy as well as a helpful summary of management of pregnancy-specific dermatoses. It will assist clinicians in the identification of specific dermatoses that pose fetal risks., Discussion: In addition to considering non-pregnancy specific conditions when approaching pruritus or a pruritic rash in pregnancy, it is important that clinicians also consider pregnancy-specific dermatoses, which have been reclassified into four categories: polymorphic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy (ICP) and atopic eruption of pregnancy. Unlike the other dermatoses, ICP begins with pruritus, and skin changes are secondary. ICP and pemphigoid gestationis are associated with fetal risks such as prematurity and stillbirth.

Bullous pemphigoid (BP) is an autoimmune blistering disease characterized by urticarial plaques and/or vesicles and tense bullae. A unique presentation of BP can occur during pregnancy, the postpartum period after delivery, or with the initiation of contraception, in which case it is referred to as pemphigoid gestationis (PG). In rare instances, newborns born to mothers with PG may also present with blisters due to transplacental passage of maternal anti-bullous pemphigoid 180 (BP180) or 230 (BP230) immunoglobulin G (IgG). In this report, we present an unusual case of neonatal PG in an infant born to an asymptomatic mother without a previous diagnosis of PG., (© 2021 Wiley Periodicals LLC.)

Subepithelial autoimmune blistering dermatoses are a group of rare skin disorders that are characterized by the disruption of the dermal-epidermal junction through the action of autoantibodies. The third article in this continuing medical education series explores the background, epidemiology, clinical features, and diagnostic criteria of each of the major subepithelial autoimmune blistering dermatoses, including bullous pemphigoid, pemphigoid gestationis, lichen planus pemphigoides, mucous membrane pemphigoid, linear IgA bullous dermatosis, and dermatitis herpetiformis., Competing Interests: Conflicts of interest Dr Murrell is an investigator/advisor for AstraZeneca, Genentech, Immune Pharmaceuticals, Principia-Biopharma, Roche, Sanofi, and a co-creator of the BPDAI & ABQOL scores. Authors Montagnon, Lehman, Camilleri, and Tolkachjov have no conflicts of interest to declare., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Pemphigoid gestationis is a rare autoimmune subepidermal bullous dermatosis occurring during pregnancy and post partum. A 32-year-old woman developed itchy urticarial wheals over the trunk and extremities at 6 months of gestation. This was not controlled with antihistamines, and 2 months later, the patient developed multiple vesiculobullous lesions. The patient had an exacerbation 3 weeks post-delivery. She did not go into remission for 6 months post partum despite treatment with prednisolone 40 mg/day, doxycycline 100 mg two times per day and dapsone 100 mg/day. The patient went into remission following treatment with three courses of intravenous immunoglobulin 2 mg/kg/course and 2 doses of rituximab 1 g at a 2-week interval., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and consensus statements was conducted in November 2019. Search terms included "guideline or guidelines" or "consensus" and "pemphigoid" or "autoimmune blistering disease" or "epidermolysis bullosa acquisita". We included guidelines from established dermatologic societies and expert consensus groups. We excluded literature reviews, guidelines established by an association without dermatologists, or those specific to a single treatment. Guidelines in all languages were considered. Eleven guidelines from dermatologic associations and consensus groups meeting our inclusion criteria were selected. Several differences between recommendations, most notably when to introduce adjuvants for refractory disease, were found in bullous pemphigoid. In mucous membrane pemphigoid, treatment was directed to the sites involved and managed with systemic corticosteroids and immunosuppressants/biologics. There was no universal consensus on the first-line treatment for epidermolysis bullosa acquisita, but a combination of immunosuppressive, anti-inflammatory, and anti-neutrophil therapy was utilized. Comparison of the management guidelines revealed underrepresentation of guidelines from developing nations and key differences between the management styles among dermatologists from Europe and Asia. We attribute these discrepancies to the time elapsed between guidelines, regional differences, and demands of the local healthcare systems.

Pemphigoid diseases are autoimmune subepidermal blistering diseases affecting the skin and mucous membranes, which are caused by autoantibodies targeting structural hemidesmosomal proteins or hemidesmosome-associated proteins. Variants of pemphigoid can be differentiated based on targeted antigens and clinical aspects. In this review, we will discuss pemphigoid variants that predominantly affect the skin, and provide clinicians with clues to diagnosis., (© 2019 British Association of Dermatologists.)

Pemphigoid gestationis (PG), also known as herpes gestationis, is the prototypic pregnancy-associated autoimmune bullous disease (AIBD), but also the other AIBDs, notably pemphigus vulgaris, may begin or exacerbate during pregnancy. Although the increase in concentration of T and B regulatory cells makes pregnancy a state of increased immunologic tolerance toward the semiallogeneic fetal antigens, a prevalent T helper (Th) 2 profile, that is reported to be associated with pregnancy, may cause exacerbation of pemphigus and AIBDs in general during this period. Active disease may lead to stillbirth, spontaneous abortion, preterm pregnancy, low birthweight, and neonatal pemphigus. PG is a rare AIBD usually starting during the third trimester of pregnancy and healing in the postpartum. It is due to the formation of autoantibodies directed against different epitopes of bullous pemphigoid (BP) 180 as a consequence of the aberrant expression of BP180 in the placental tissue of genetically predisposed women. PG is characterized by vesicles with herpetiform distribution, blisters and urticarial elements typically involving the periumbilical area and the distal portion of the upper limbs. Diagnosis is based on: 1) physical examination; 2) histopathological pattern consisting of a dermal inflammatory infiltrate rich in eosinophils; 3) direct immunofluorescence test demonstrating linear deposits of complement fraction 3 and immunoglobulin G along the basement membrane zone; 4) detection of circulating autoantibodies by means of indirect immunofluorescence or enzyme linked immunosorbent assay. Here, we provide an updated overview on the pathophysiologic mechanisms of pregnancy-associated or pregnancy-exacerbated AIBDs, focusing also on peculiar clinical features of these disorders.

Pruritus in pregnancy can be a source of significant discomfort in the pregnant patient. Some cases are associated with pregnancy-specific dermatoses, although some patients experience a flare of a preexisting dermatosis. Severe pruritus may be a manifestation of a pregnancy-specific dermatosis associated with increased fetal risks and complications. Early accurate diagnosis and appropriate management are important. Examination often reveals important clinical findings, aiding accurate diagnosis. Pemphigoid gestationis often presents with periumbilical involvement, whereas polymorphic eruption of pregnancy spares the umbilicus and presents in the striae distensae. Intrahepatic cholestasis of pregnancy is associated with intense pruritus of the palms., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Pemphigoid gestationis is a skin-specific autoimmune disorder that can sometimes present as the cutaneous manifestation of a multiorgan disease due to potentially common pathogenic mechanisms. We report a severe form of pemphigoid gestationis in a 32-year-old primigravida woman, who presented at 22 weeks of gestation with headaches and blurred vision, later developing encephalitis, intrauterine fetal demise and dilated cardiomyopathy., (© 2017 The Australasian College of Dermatologists.)

Pemphigoid gestationis (PG), also known as herpes gestationis , is a rare autoimmune blistering disease specific to pregnancy, which usually presents in the second or third trimesters and, in 15%-25% of cases, during the immediate postpartum period. 1 Although the ethiopathogeny of PG is not fully clarified, most patients develop antibodies against a 180 kDa transmembrane hemidesmosomal protein (BP180; BPAG2; collagen XVII). 2 PG has a strong association with human leucocyte antigens DR3 and DR4. 3 We report a case of a 29-year-old female patient with PG successfully treated with intravenous immunoglobulin., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Lichen planus pemphigoides (LPP) is a rare condition characterized by tense blisters that arise on lesions of lichen planus (LP) and on unaffected skin. We present the case of a 25-year-old pregnant woman at 12 weeks' gestation who developed an acute bullous eruption after 5 months of worsening LP. Similarities to pemphigoid gestationis (PG) included lesions around the periumbilical area and multiple urticarial erythematous papules and plaques in addition to linear C3 and IgM deposition along the basement membrane zone (BMZ) on direct immunofluorescence (DIF).

Pemphigoid gestationis (PG) is the only autoimmune disease exclusively emerging in pregnancy. It belongs to the pemphigoid group of disorders, a class of autoimmune blistering skin diseases featuring an immune response against different hemidesmosomal proteins. PG is caused by a break of immunotolerance against the hemidesmosomal protein BP180. Several lines of evidence suggest that this break of immunotolerance is linked to specific maternal major histocompatibility complex (MHC) class II gene variants and aberrant expression of MHC class II molecules in the placenta. The close time association of the emergence of PG with pregnancy and the obviously very short period required from the initial break of immunotolerance to the onset of skin inflammation set PG into a unique position among autoimmune diseases in view of the fact that, for other autoimmune diseases, the time and site of the break of immunotolerance are usually vastly elusive and the period of silent disease can only be speculated on. In this review we highlight the features of PG and summarize current knowledge about its pathogenesis. We believe that this disease offers the best opportunity to elucidate comprehensively all phases of the pathogenesis of an autoantibody-driven disease., (Copyright © 2016 Elsevier Inc. All rights reserved.)